How can I be sure that the patient has a biliary stricture?

The constellation of symptoms and the resulting diagnosis of bile duct stricture are largely driven by the etiology of the condition. Presenting symptoms may include abdominal pain, pruritus, nausea and vomiting, acholic (clay-colored) stools, dark urine, and recurrent fevers.

Bile duct strictures may be categorized broadly into two categories: (1) benign biliary strictures and (2) malignant biliary strictures. The majority of benign biliary strictures are iatrogenic, resulting from operative trauma. In terms of frequency, biliary strictures resulting from laparoscopic cholecystectomy are more common than open cholecystectomy, followed by occurrence at the site of biliary anastomosis after hepatic resection or liver transplantation. Injuries include cutting or transecting the common hepatic or common bile duct; excessive use of cautery, resulting in thermal injury; inaccurate placement of clips or sutures; excessive traction on the gallbladder neck; or vascular compromise with resultant ischemic injury.

Pancreatic cancer, surrounding and compressing the distal bile duct, is the most common cause of malignant biliary strictures (>90%). Gallbladder cancer, portal adenopathy from metastatic disease, and cholangiocarcinoma comprise the majority of the remaining 10% of cases.

Biliary strictures may be relatively asymptomatic, such as in the patient with pancreatic cancer presenting with painless jaundice; however, if untreated, these may result in life-threatening complications, such as ascending cholangitis, hepatic abscess, and secondary biliary cirrhosis.

A tabular or chart listing of features and signs and symptoms

What are the clues?

Table I summarizes features and signs and symptoms of biliary strictures.

Table I.

Clinical features and signs and symptoms of biliary strictures

Malignant

Benign

Weight loss

Recent surgery: cholecystectomy, liver resection, liver transplant

Acute rise in LFTs

No history of abdominal surgery

History of IBD: UC or Crohn’s disease

Recent worsening of PSC

Fluctuating LFTs

Painless jaundice

Prior choledocholithiasis

History of prior malignancy (e.g., metastatic disease)

Trauma

How can I confirm the diagnosis?

Initial testing should include serology for alkaline phosphatase as well as a total and direct serum bilirubin. The serum alkaline phosphatase level will be elevated in patients with partial bile duct obstruction and may be more than three times the upper limit of normal in patients with clinical evidence of jaundice.

A right upper-quadrant ultrasound may be useful to detect dilated bile ducts suggesting extrahepatic obstruction.

Endoscopic retrograde cholangiopancreatography (ERCP) should be reserved as the final confirmatory diagnostic test and should be the last resort if MRI/MRCP or CT scanning does not provide sufficient detail of the biliary tree for definitive diagnosis, or for those patients for whom a therapeutic procedure is anticipated.

An algorithm for managing biliary strictures is shown in Figure 1.

Figure 1.

Algorithm for management of biliary strictures.

What other diseases, conditions, or complications should I look for in patients with biliary strictures?

Differential diagnoses

Differential diagnoses are summarized in Table II.

Table II.

Differential diagnoses

Malignant strictures

Benign strictures

Pancreatic adenocarcinoma

Iatrogenic

Lymphoma

Laparoscopic cholecystectomy (0.4%-0.6%)

Gallbladder cancer

Open cholecystectomy (0.2%-0.3%)

Cholangiocarcinoma

Liver resection (malignancy, trauma, benign disease)

Hepatocellular carcinoma

Liver transplant

Metastatic disease

Anastomotic stenosis

Portal adenopathy from metastases

Ischemic injury

Hepatic artery thrombosis

Chronic pancreatitis

Autoimmune pancreatitis

Primary sclerosing cholangitis

Autoimmune cholangiopathy

HIV cholangiopathy

Secondary sclerosing cholangitis

Biliary papillomatosis

Complications of endoscopic and percutaneous management of biliary strictures

Complications of endoscopic and percutaneous management of biliary strictures are summarized in Table III.

Table III.

Percutaneous and endoscopic management complications

Percutaneous transhepatic drainage

Endoscopic drainage

Sepsis

5%

Sepsis

<1%

Bleeding

5%

Bleeding

1.3%

Infection (abscess)

5%

Cholecystitis

0.2%

Bile leak/peritonitis

5%

Cholangitis

<1%

Cholecystitis

5%

Pancreatitis

3.5%

Pancreatitis

5%

Ductal perforation

0.1%-0.6%

Pneumothorax/pleural injury/effusion

2%

Luminal perforation

0.1%

Death

3%

Death

0.2%-0.4%

What is the right therapy for the patient with biliary stricture?

Biliary obstruction can be managed endoscopically, percutaneously, or surgically (Table IV). The least invasive and least morbid approach must be weighed and selected for each patient based on the presenting clinical scenario. For example, the patient presenting with painless jaundice due to a resectable pancreatic head mass or ampullary malignancy may be best treated with definitive therapy: immediate surgical resection. On the other hand, the patient with a borderline resectable malignancy or jaundice complicated by cholangitis should proceed directly to endoscopic stent placement. Percutaneous drainage should be reserved for those patients in whom endoscopic therapy fails, those with surgically altered anatomy preventing access to the ampulla (gastric bypass, long Roux-en-y limbs), or complete biliary obstruction (transection of the bile duct).

Table IV.

Management therapies for biliary obstructions

Endoscopic therapy (preferred)

Percutaneous therapy

Surgical therapy

Intact anatomy

Failed endoscopic approach

Pancreatic mass resectable

Ascites present

Altered anatomy: Roux-en-y, gastric bypass

Ampullary mass resectable

Coagulopathy

Complete biliary obstruction

Biliary bypass when enteric bypass also needed (GOO)

Small ducts (PSC with dominant stricture)

Complete gastric outlet obstruction (bridge to surgery)

Failed percutaneous approach

What is the most effective initial therapy?

Endoscopic biliary stent placement is the preferred initial therapy for most biliary strictures. The approach to benign strictures usually includes a trial of endoscopic stenting to attempt to improve or resolve the stricture.

In most patients with benign strictures of the extrahepatic ducts, a trial using either multiple plastic stents, rather than a single stent, is more effective in long-term stricture resolution. This approach is most commonly considered in those with post-operative biliary strictures and in those with biliary obstruction due to chronic pancreatitis, and rarely used in those with primary sclerosing cholangitis. Multiple stents are usually placed and changed every 3 to 6 months, with a treatment duration of 6 to 12 months. Failure of the stricture to resolve is an indication for surgical therapy.

Recent studies suggest that fully covered metal stents may be equivalent to the approach using multiple plastic stents, but this is not yet a standard approach. Temporary biliary stenting may be used in those with primary sclerosing cholangitis and in those who have a dominant extrahepatic stricture; however, care must be taken to rule out malignancy in these patients. In general, balloon dilation rather than stenting is preferred in patients with primary sclerosing cholangitis.

Failure of endoscopic stent therapy for benign biliary strictures is an indication for surgical biliary bypass. Biliary strictures due to chronic pancreatitis are particularly prone to fail endoscopic therapy. Surgery usually requires a choledochojejunostomy utilizing a defunctionalized Roux limb, although occasionally, a choledochoduodenostomy is performed based on surgical preference and stricture location.

Malignant strictures of the distal bile duct are usually treated with placement of a covered or uncovered self-expanding metal stent. More proximal malignant biliary strictures (cholangiocarcinoma) are the most difficult to manage, often require multiple plastic or metal stents, and, not infrequently, require additional percutaneous biliary drains to facilitate biliary drainage.

Listing of usual initial therapeutic options, including guidelines for use, along with expected result of therapy.

N/A

A listing of a subset of second-line therapies, including guidelines for choosing and using these salvage therapies

While endoscopic stent placement is often used for managing benign and malignant biliary strictures, this technique requires frequent stent changes and may be complicated by episodes of stent occlusion, producing biliary pain, jaundice, or cholangitis.

Plastic biliary stents occlude rapidly, and no more than 3 months should pass between planned stent changes. Metal stents may also occlude, either due to tissue ingrowth or luminal occlusion for sludge or stones.

Listing of these, including any guidelines for monitoring side effects.

See Figure 1 for a therapeutic algorithm for managing biliary strictures.

How should I monitor the patient with biliary stricture?

The major determinant of mortality in patients with bile duct strictures is the underlying disease condition. Management of bile duct strictures due to malignancy is based on the etiology of the malignancy and the overall prognosis. For example, plastic stents are preferable in patients with large tumors (>3 cm) or those due to liver metastases, as these patients have a life expectancy of 3 to 4 months, and plastic stents are effective for this duration and are more cost effective.

For those patients with an expected survival of more than 6 months, self-expanding metal stents (SEMS) are preferred and are more cost effective. Benign biliary strictures due to operative injury, radiation, trauma, or chronic pancreatitis generally have a good prognosis.

Multiple procedures combining biliary dilation and placement of multiple plastic stents have been demonstrated to be effective management of benign strictures. While use of SEMS for management of benign strictures remains controversial, there may be a role for use of fully covered self-expanding metal stents in cases with refractory strictures. Response to endoscopic therapy of refractory biliary strictures due to chronic pancreatitis has been poor.